Cylindrical Bronchiectasis (Tubular Bronchiectasis)

In cylindrical (tubular) bronchiectasis, the bronchi are dilated and rigid and have regular outlines similar to a tube. X-ray examination shows that the dilated bronchi fail to taper for 6 to 10 generations and then appear to end abruptly because of mucous obstruction (see Figure 13-1, B).

Varicose Bronchiectasis (Fusiform Bronchiectasis)

In varicose (fusiform) bronchiectasis, the bronchi are dilated and constricted in an irregular fashion similar to varicose veins, ultimately resulting in a distorted, bulbous shape (see Figure 13-1, A).

Cystic Bronchiectasis (Saccular Bronchiectasis)

In cystic (saccular) bronchiectasis, the bronchi progressively increase in diameter until they end in large, cystlike sacs in the lung parenchyma. This form of bronchiectasis causes the greatest damage to the tracheobronchial tree. The bronchial walls become composed of fibrous tissue alone—cartilage, elastic tissue, and smooth muscle are all absent (see Figure 13-1, C).

The following are the major pathologic or structural changes associated with bronchiectasis:

Bronchiectasis is commonly seen in individuals who have recurrent and prolonged episodes of lower respiratory tract infections (e.g., pneumonia, tuberculosis, and fungal infections). For example, children who have frequent bouts of bronchopneumonia—because of the respiratory complications of measles, chickenpox, whooping cough, or influenza—may acquire some form of bronchiectasis later in life.

It is estimated that cystic fibrosis causes approximately 50% of the bronchiectasis cases in the United States today. Because of the impairment of the mucociliary clearance mechanism—and the abundance of stagnant, thick mucus—associated with cystic fibrosis, bronchial obstruction from mucous plugging and bronchial wall infection frequently result. The necrotizing inflammation that develops under these conditions often leads to secondary bronchiectasis.

Systemic Disorders

Bronchiectasis is associated with several systemic conditions such as rheumatologic disorders, inflammatory bowel disease, and acquired immunodeficiency syndrome (AIDS).

 OVERVIEW of the Cardiopulmonary Clinical Manifestations Associated with Bronchiectasis

The following clinical manifestations result from the pathophysiologic mechanisms caused (or activated) by Excessive Bronchial Secretions (see Figure 9-12), Bronchospasm (see Figure 9-11), Atelectasis (see Figure 9-8), Consolidation (see Figure 9-9), and Increased Alveolar-Capillary Membrane Thickness) (See Figure 9-10)—the major anatomic alterations of the lungs associated with bronchiectasis (see Figure 13-1).

CLINICAL DATA OBTAINED AT THE PATIENT’S BEDSIDE

Depending on the amount of bronchial secretions and the degree of bronchial destruction and fibrosis associated with bronchiectasis, the disease may create an obstructive or a restrictive lung disorder or a combination of both. If the majority of the bronchial airways are only partially obstructed, the bronchiectasis manifests primarily as an obstructive lung disorder. If, on the other hand, the majority of the bronchial airways are completely obstructed, the distal alveoli collapse, atelectasis results, and the bronchiectasis manifests primarily as a restrictive disorder. Finally, if the disease is limited to a relatively small portion of the lung—as it often is—the patient may not have any of the following clinical manifestations.

The Physical Examination

Vital Signs

Increased Respiratory Rate (Tachypnea)

Several pathophysiologic mechanisms operating simultaneously may lead to an increased ventilatory rate:

• Stimulation of peripheral chemoreceptors (hypoxemia)

• Decreased lung compliance and increased ventilatory rate relationship

• Anxiety

Increased Heart Rate (Pulse) and Blood Pressure

Use of Accessory Muscles during Inspiration

Use of Accessory Muscles during Expiration

Pursed-Lip Breathing (When Primarily Obstructive in Nature)

Increased Anteroposterior Chest Diameter (Barrel Chest) (When Primarily Obstructive in Nature)

Cyanosis

Digital Clubbing

Peripheral Edema and Venous Distention

Because polycythemia and cor pulmonale are associated with severe bronchiectasis, the following may be seen:

• Distended neck veins

• Pitting edema

• Enlarged and tender liver

Cough, Sputum Production, and Hemoptysis

Chronic cough with production of large quantities of foul-smelling sputum is a hallmark of bronchiectasis. A 24-hour collection of sputum is usually voluminous and tends to settle into several different layers. Streaks of blood are seen frequently in the sputum, presumably originating from necrosis of the bronchial walls and erosion of bronchial blood vessels. Frank hemoptysis may also occur from time to time, but it is rarely life threatening. Because of the excessive bronchial secretions, secondary bacterial infections are frequent. Haemophilus influenzae, Streptococcus, Pseudomonas aeruginosa, and various anaerobic organisms are commonly cultured from the sputum of patients with bronchiectasis.

The productive cough in bronchiectasis is triggered by the large amount of secretions that fill the tracheobronchial tree. The stagnant secretions stimulate the subepithelial mechanoreceptors, which in turn produce a vagal reflex that triggers a cough. The subepithelial mechanoreceptors are found in the trachea, bronchi, and bronchioles, but they are predominantly located in the upper airways.

Chest Assessment Findings

When the bronchiectasis is primarily obstructive in nature:

• Decreased tactile and vocal fremitus

• Hyperresonant percussion note

• Diminished breath sounds

• Wheezing

• Rhonchi and wheezing

When the bronchiectasis is primarily restrictive in nature (over areas of atelectasis and consolidation):

• Increased tactile and vocal fremitus

• Bronchial breath sounds

• Crackles

• Whispered pectoriloquy

• Dull percussion note

CLINICAL DATA OBTAINED FROM LABORATORY TESTS AND SPECIAL PROCEDURES

Pulmonary Function Test Findings

Moderate to Severe Bronchiectasis (When Primarily Obstructive Lung Pathophysiology)

FORCED EXPIRATORY FLOW RATE FINDINGS

FVC	FEVT	FEV1/FVC ratio	FEF25%-75%
↓	↓	↓	↓
FEF50%	FEF200-1200	PEFR	MVV
↓	↓	↓	↓

LUNG VOLUME AND CAPACITY FINDINGS

VT	IRV	ERV	RV
N or ↑	N or ↓	N or ↓	↑
VC	IC	FRC	TLC	RV/TLC ratio
↓	N or ↓	↑	N or ↑	N or ↑

Pulmonary Function Test Findings

Moderate to Severe Bronchiectasis (When Primarily Obstructive Lung Pathophysiology)

FORCED EXPIRATORY FLOW RATE FINDINGS

FVC	FEVT	FEV1/FVC ratio	FEF25%-75%
↓	N or ↓	N or ↑	N or ↓
FEF50%	FEF200-1200	PEFR	MVV
N or ↓	N or ↓	N or ↓	N or ↓

LUNG VOLUME AND CAPACITY FINDINGS

VT	IRV	ERV	RV
N or ↓	↓	↓	↓
VC	IC	FRC	TLC	RV/TLC ratio
↓	↓	↓	↓	N

Arterial Blood Gases

Bronchiectasis

MILD TO MODERATE STAGES

Acute Alveolar Hyperventilation with Hypoxemia (Acute Respiratory Alkalosis)

pH	Paco2		Pao2
↑	↓	↓ (slightly)	↓

SEVERE STAGE

Chronic Ventilatory Failure with Hypoxemia (Compensated Respiratory Acidosis)

pH	Paco2		Pao2
N	↑	↑ (significantly)	↓

Acute Ventilatory Changes Superimposed on Chronic Ventilatory Failure

Because acute ventilatory changes are frequently seen in patients with chronic ventilatory failure, the respiratory care practitioner must be familiar with and alert for the following:

• Acute alveolar hyperventilation superimposed on chronic ventilatory failure, and/or

• Acute ventilatory failure (acute hypoventilation) superimposed on chronic ventilatory failure

Oxygenation Indices* Bronchiectasis

Moderate to Severe Stages

	Do2‡		C(a-)o2	O2ER
↑	↓	N	N	↑	↓

^‡The Do₂ may be normal in patients who have compensated to the decreased oxygenation status with (1) an increased cardiac output, (2) an increased hemoglobin level, or (3) a combination of both. When the Do₂ is normal, the O₂ER is usually normal.

---

^*C(a-)O₂, Arterial-venous oxygen difference; DO₂, total oxygen delivery; O₂ER, oxygen extraction ratio; , pulmonary shunt fraction; , mixed venous oxygen saturation; , oxygen consumption.

Hemodynamic Indices†

Bronchiectasis Moderate to Severe Stages

CVP	RAP		PCWP	CO	SV
↑	↑	↑	N	N	N
SVI	CI	RVSWI	LVSWI	PVR	SVR
N	N	↑	N	↑	N

---

^†CO, Cardiac output; CVP, central venous pressure; LVSWI, left ventricular stroke work index; , mean pulmonary artery pressure; PCWP, pulmonary capillary wedge pressure; PVR, pulmonary vascular resistance; RAP, right atrial pressure; RVSWI, right ventricular stroke work index; SV, stroke volume; SVI, stroke volume index; SVR, systemic vascular resistance.

ABNORMAL LABORATORY TESTS AND PROCEDURES

Hematology

• Increased hematocrit and hemoglobin

• Elevated white blood count (WBC) if patient is acutely infected

Sputum Examination

• Streptococcus pneumoniae

• Haemophilus influenzae

• Pseudomonas aeruginosa

• Anaerobic organisms

RADIOLOGIC FINDINGS

Chest Radiograph

When the Bronchiectasis Is Primarily Obstructive in Nature

• Translucent (dark) lung fields

• Depressed or flattened diaphragms

• Long and narrow heart (pulled down by diaphragms)

• Enlarged heart

• Areas of consolidation and/or atelectasis may or may not be seen

When the pathophysiology of bronchiectasis is primarily obstructive in nature, the lungs become hyperinflated, leading to an increased functional residual capacity and depressed diaphragms. Because right and left ventricular enlargement and failure may develop as secondary problems during the advanced stages of bronchiectasis, an enlarged heart may be seen on the chest radiograph.

Although the chest radiograph is not be as valuable as the computed tomography (CT) scan in identifying a specific type of bronchiectasis (i.e., cystic, varicose, or cylindrical), a careful analysis of chest radiographs usually reveals abnormalities in the majority of the cases. Figure 13-2, for example, shows a patient with gross cystic bronchiectasis and overinflated lungs.

When the Bronchiectasis Is Primarily Restrictive in Nature

• Atelectasis and consolidation

• Infiltrates (suggesting pneumonia)

• Increased opacity

In generalized bronchiectasis, such as commonly seen in cystic fibrosis, there is usually overinflation of the lungs. However, when the bronchiectasis is localized, the chest radiograph often reveals a restrictive pathology such as atelectasis, consolidation, or infiltrates. When atelectasis and consolidation develop as a result of bronchiectasis, an increased opacity and reduced lung volume are seen in these areas on the radiograph. For example, Figure 13-3 illustrates a marked volume loss in a patient with left lower lobe bronchiectasis. Figure 13-4 shows a patient with Kartagener’s syndrome with severe volume loss.

Bronchogram

Bronchography (the injection of an opaque contrast material into the tracheobronchial tree) is occasionally performed on patients with bronchiectasis. Bronchograms may be useful in diagnosing bronchiectasis and delineating the extent and type of tracheobronchial involvement. In cylindrical bronchiectasis, the bronchogram shows dilated, cylinder-shaped bronchioles (Figure 13-5). In cystic bronchiectasis, the bronchogram shows large, saclike structures; fibrotic markings; associated atelectasis; and adjacent emphysema (Figure 13-6). In varicose bronchiectasis, the bronchogram may show bronchi that are dilated and constricted in an irregular fashion and terminate in a distorted, bulbous shape (Figure 13-7). CT of the chest has largely replaced this technique.

Computed Tomography (CT Scan)

Increased bronchial wall opacity is often seen. The bronchial walls may appear as follows:

• Thick

• Dilated

• Characterized by ring lines or clusters

• Signet ring–shaped

• Flame-shaped

The CT scan changes may include many findings that are similar to those seen on the chest radiograph. The bronchial walls may appear thick, dilated, or as rings of opacities arranged in lines or clusters. A characteristic appearance in bronchiectasis is the end-on signet ring opacity produced by the ring shadow of a dilated airway with its accompanying artery (Figure 13-8).

The specific type of bronchiectasis can be confirmed with the CT scan. For example, Figure 13-9 confirms the presence of cylindrical bronchiectasis. Figure 13-10 shows varicose bronchiectasis and Figure 13-11 shows cystic bronchiectasis. Airways that are filled with secretions produce rounded or flame-shaped opacities that can be identified by following them through adjacent sections to unfilled airways. The CT scan also confirms atelectasis, consolidation, fibrosis, scarring, and hyperinflation.

Oxygen therapy is used to treat hypoxemia, decrease the work of breathing, and decrease myocardial work. The hypoxemia that develops in bronchiectasis is usually caused by the pulmonary shunting associated with the disorder. When the patient demonstrates chronic ventilatory failure during the advanced stages of bronchiectasis, caution must be taken not to overoxygenate the patient (see Oxygen Therapy Protocol, Protocol 9-1).

Expectorants sometimes are ordered when oral liquids and aerosol therapy alone are not sufficient to facilitate expectoration (see Appendix II, Expectorants). Their clinical effectiveness is doubtful.

Respiratory Assessment and Plan

The patient was treated vigorously with chest physiotherapy and mucolytic therapy. The physician prescribed antibiotics and administered pneumonia vaccine. The patient was discharged from the hospital after 3 days with considerable improvement. He was instructed to seek prompt medical attention for all future pulmonary infections. His wife was instructed in postural drainage techniques.

Approximately 6 months later, the patient arrived at the emergency department complaining of a productive cough, pain on the left side of the chest (made worse by deep breathing), shaking chills and fever for 3 days, and noticeable swelling of both ankles. Since his previous visit, he had been performing CPT and PD only “once or twice a week,” had gained 30 pounds, and had taken a new job as a painter’s apprentice. He admitted to smoking an occasional cigarette. There had been no known infectious disease exposure.

Physical examination revealed a young man in obvious respiratory distress. His vital signs were blood pressure 160/100, heart rate 110 bpm and regular, respiratory rate 20/min, and oral temperature 101.5° F. His sputum was foul-smelling (a fecal odor), thick, and yellow-green. His cough was strong. Auscultation revealed sibilant rhonchi and crackles over both bases. There was mild clubbing of fingers and toes. The physician wrote “bronchiectasis” in the working diagnosis section of the patient’s chart.

Although a chest x-ray film had been taken, it was not yet available. The patient’s WBC was 23,500 mm³, with 80% segmented neutrophils and 10% bands. Room air ABG showed pH 7.51, Paco₂ 28 mm Hg, 21, and Pao₂ 45  mm Hg. His Spo₂ at rest on room air was 86%; it fell to 78% when he got out of bed to go to the bathroom. The respiratory care practitioner recorded the following note in the patient’s emergency department chart.

Respiratory Assessment and Plan

Discussion

The main challenge facing the respiratory care practitioner caring for the patient with bronchiectasis is one of efficient removal of excessive bronchopulmonary secretions. Over the years, postural drainage and percussion, good systemic hydration, and judicious use of antibiotics have been the hallmarks of therapy. More recently, intermittent use of mucolytics, percussive ventilation, and Lung Expansion Therapy (see Protocol 9-3) has become more common. Pneumococcal prophylaxis is, of course, important, as is prompt attention to parenchymal pulmonary infections such as pneumonia. The clinical distinction between chronic bronchiectasis and cystic fibrosis is a subtle one at the bedside, and the latter condition must always be ruled out in patients with bronchiectasis. The goal of long-term therapy in bronchiectasis is prevention of lung parenchyma–destroying pulmonary infections and avoidance of frequent hospitalizations. Hemoptysis is often a sign of more deep-seated infection requiring antibiotic therapy.

The clinical manifestations throughout this case were all based on the clinical scenario associated with excessive airway secretions. For example, the thick yellow sputum resulted in decreased ratios, venous admixture, and hypoxemia. These pathophysiologic mechanisms caused clinical manifestations of an increase in blood pressure and heart rate, acute alveolar hyperventilation with moderate hypoxemia, and rhonchi.

Digital clubbing associated with hypoxemia is another clinical manifestation of bronchiectasis. After the first assessment, both the Oxygen Therapy Protocol and Bronchopulmonary Hygiene Therapy Protocol were administered appropriately (see Protocols 9-1 and 9-2). The therapist’s review of the chest x-ray allowed him to target the postural drainage therapy. Low-flow oxygen per nasal cannula, aerosolized bronchodilators (albuterol) and mucolytic medication (acetylcysteine), chest percussion, and postural drainage therapy were selected from these protocols and applied with good results.

Finally, during the second admission, patient noncompliance was evident (i.e., weight gain, resumption of smoking, employment in a dusty workplace, failure to continue CPT and PD), which further complicated the patient’s respiratory disorder. In response to the patient’s condition, the whole respiratory care regimen was upgraded by an increase in frequency of treatments, with a strong emphasis on the patient’s responsibility for his own care.